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Comprehensive analyses and characterization of haemophagocytic lymphohistiocytosis in Vietnamese children

✍ Scribed by Lam T. My; Le B. Lien; Wen-Chuan Hsieh; Toshihiko Imamura; Tran N. K. Anh; Phan N. L. Anh; Nguyen T. Hung; Fan-Chen Tseng; Chia-Yu Chi; Ngo T. H. Dao; Duong T. M. Le; Le Q. Thinh; Tran T. Tung; Shinsaku Imashuku; Tang C. Thuong; Ih-Jen Su

Book ID
108676558
Publisher
John Wiley and Sons
Year
2010
Tongue
English
Weight
172 KB
Volume
148
Category
Article
ISSN
0007-1048

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Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal-recessive disease that affects young children. It presents as a severe hyperinflammatory syndrome with activated macrophages and T lymphocytes. Mutations in the perforin 1 gene (PRF1) were found in FHL-2 in 15-50% of all cases. Defect